Twelve tips for optimising learning for postgraduate doctors in the operating theatre.

Learning in the operating theatre forms a critical part of postgraduate medical education. Postgraduate doctors present a diverse cohort of learners with a wide range of learning needs that will vary by their level of experience and curriculum requirements. With evidence of both trainee dissatisfaction with the theatre learning experience and reduced time spent in the operating theatre, which has been exacerbated by the effects of the Covid-19 pandemic, it is vital that every visit to the operating theatre is used as a learning opportunity. We have devised 12 tips aimed at both learners and surgeons to optimise learning in the operating theatre, set out into four domains: educational context, preparation, learning in theatre, feedback and reflection. These tips have been created by a process of literature review and acknowledgment of established learning theory, with further discussion amongst surgical trainees, senior surgical faculty, surgical educators and medical education faculty.

PIEZO2-related distal arthrogryposis type 5: Longitudinal follow-up of a three-generation family broadens phenotypic spectrum, complications, and health surveillance recommendations for this patient group.

Arthrogryposis is a heterogenous condition with a wide variety of etiological causes. It has been subdivided clinically based on the presence of additional features. Dominant gain of function (GoF) pathogenic variants in PIEZO2 have been associated with several forms of arthrogryposis. Previous reports have focused on diagnosis and clinical features. We report a three-generation family with four affected individuals with a known pathogenic GoF change p.(Glu2727del) in PIEZO2. All family members presented at birth with distal arthrogryposis and ophthalmoplegia but have varied in their subsequent clinical course with differences in mobility and joint restriction. In the longer term, other features have presented including dysphagia, back pain and spinal stenosis-like symptoms, raised intraocular pressure, and progressive restrictive lung disease. As far as we know, this is the first report detailing the longitudinal follow-up of a three-generation family which highlights potential long-term complications in patients with PIEZO2-related arthrogryposis. We present this family to demonstrate the importance of long-term follow-up for the clinical management of this group of patients.

A recurrent pathogenic variant in TPM2 reveals further phenotypic and genetic heterogeneity in multiple pterygium syndrome-related disorders.

Multiple pterygium syndrome (MPS) disorders are a phenotypically and genetically heterogeneous group of conditions characterized by multiple joint contractures (arthrogryposis), pterygia (joint webbing) and other developmental defects. MPS is most frequently inherited in an autosomal recessive fashion but X-linked and autosomal dominant forms also occur. Advances in genomic technologies have identified many genetic causes of MPS-related disorders and genetic diagnosis requires large targeted next generation sequencing gene panels or genome-wide sequencing approaches. Using the Illumina TruSightOne clinical exome assay, we identified a recurrent heterozygous missense substitution in TPM2 (encoding beta tropomyosin) in three unrelated individuals. This was confirmed to have arisen as a de novo event in the two patients with parental samples. TPM2 mutations have previously been described in association with a variety of dominantly inherited neuromuscular phenotypes including nemaline myopathy, congenital fibre-type disproportion, distal arthrogryposis and trismus pseudocamptodactyly, and in a patient with autosomal recessive Escobar syndrome and a nemaline myopathy. The three cases reported here had overlapping but variable features. Our findings expand the range of TMP2-related phenotypes and indicate that de novo TMP2 mutations should be considered in isolated cases of MPS-related conditions.

Multiple long bone cysts revealed by MRI in trichorhinophalangeal syndrome type II predisposing to pathological fractures.

Trichorhinophalangeal syndrome type II is a rare genetic disorder with the few published case reports mainly reporting the radiographic skeletal manifestations. There are no published imaging reports of long bone cysts involving multiple bones in this condition. We report a unique case of bone cysts involving multiple long bones detected with MRI in a patient with trichorhinophalangeal syndrome type II complicated by a subsequent pathological fracture. It is possible that the bone cysts are a previously undescribed feature of this syndrome; however, the evidence is insufficient to establish a definite association. Chromosomal abnormality identified in this patient is consistent with trichorhinophalangeal syndrome type II with no unusual features. Although the nature of these bone cysts is unclear, they are one of the causes of the known increased fracture risk observed in this syndrome.

Exostoses, enchondromatosis and metachondromatosis; diagnosis and management.

We describe a 5 years old girl who presented to the multidisciplinary skeletal dysplasia clinic following excision of two bony lumps from her fingers. Based on clinical examination, radiolographs and histological results an initial diagnosis of hereditary multiple exostosis (HME) was made. Four years later she developed further lumps which had the radiological appearance of enchondromas. The appearance of both exostoses and enchondromas suggested a possible diagnosis of metachondromatosis. Genetic testing revealed a splice site mutation at the end of exon 11 on the PTPN11 gene, confirming the diagnosis of metachondromatosis. While both single or multiple exostoses and enchondromas occur relatively commonly on their own, the appearance of multiple exostoses and enchondromas together is rare and should raise the differential diagnosis of metachondromatosis. Making this diagnosis is important as the lesions in metachondromatosis may spontaneously resolve and therefore surgical intervention is often unnecessary. We discuss the diagnostic findings, genetic causes, treatment and prognosis of this rare condition of which less than thirty cases have previously been reported.

UK CoPACK Study: knowledge and confidence of healthcare workers in using personal protective equipment and related anxiety levels during the COVID-19 pandemic.

BACKGROUND: Healthcare workers (HCWs) are at increased risk of coronavirus 2019 (COVID-19) infection. Personal protective equipment (PPE) and infection control guidelines help limit transmission. However, poor confidence leads to higher levels of anxiety rates and infection. We assessed knowledge and confidence in PPE among HCWs and associated anxiety. METHODS: A cross-sectional, multi-centre survey using a validated questionnaire assessing actual and self-perceived knowledge on PPE was distributed among HCWs across the UK. Confidence in PPE and levels of anxiety were assessed using the General Anxiety Disorder-7 (GAD-7) tool. RESULTS: In total, 1,055 responses were received; 99% had familiarity with PPE guidance; however, only 15% correctly answered questions on PPE guidance; 86% and 80% had received mask-fitting and donning-doffing training, respectively; 33% indicated poor/very poor hospital communication. Confidence and anxiety were related to: profession; comorbidities; self-perceived knowledge; and PPE training and communication. CONCLUSION: Confidence in PPE was poor and anxiety was related to inadequate information and training. Thus, improved communication is required for effective response to subsequent COVID-19 waves and similar pandemics.

Minimizing the need for high dependency unit support in adolescent idiopathic scoliosis surgery. Is enhanced recovery and the multidisciplinary team key?

Current trends in the surgical treatment of patients with adolescent idiopathic scoliosis (AIS) involve the use of high dependency unit (HDU) in the postoperative period. The British Scoliosis Society also recommends the availability of HDU support in the postoperative period for these patients. However, this practice may lead to unexpected theatre cancellations due to lack of availability of HDU bed on the day of surgery. We also hypothesize that this practice may eventually lead to longer inpatient stay for the patients. All AIS patients at our unit are managed on a paediatric ward postoperatively, without HDU support. The primary aim of the study therefore is to evaluate whether operating on AIS patients without HDU support is well tolerated practice. Secondary aims were to evaluate patient related outcomes, including length of stay (LOS), and postoperative analgesia requirements. Adolescents aged 12-17 years with idiopathic scoliosis deformity who were treated with posterior instrumented scoliosis (PIS) correction were included in this prospective cohort study. The study period was between 12 November 2012 and 6 August 2018. Twenty-two patients were included in the HDU group and 33 patients in the non-HDU group. These were two matched cohort groups. Data were collected on complication rates, LOS, analgesic requirements, time to bowel opening, and attainment of physiotherapy goals in the immediate postoperative period. Statistical analysis was performed using statistical software R (3.4.3). There were no complications in the non-HDU group and one pneumothorax in the HDU group. There was a significant reduction in the LOS from 7.4 days (SD ±2.3, CI 0.012) days, to 5.8 (SD ±1.4, CI 0.01) days in the non-HDU group (P = 0.0001). There was no significant difference statistically or clinically in opiate usage between the HDU group, 115 mg (SD ±60.7, CI 0.8) and the non-HDU group 116 mg (SD ±55.8, CI 0.6) (P = 0.609). However, there was an improvement in pain scores in the non-HDU group where oral analgesics only were used (P = 0.002). A cost saving of £2038.80 per AIS case was made in the non-HDU group. AIS surgery can be performed safely without the need for HDU support in healthy adolescents. An oral analgesia-based enhanced recovery regime compares favourably to patient-controlled analgesia (PCA) and indicates these patients can be managed safely with strong multidisciplinary support on a paediatric ward.

A new unilateral abduction orthosis for Ponseti-treated clubfoot: A cohort study to assess compliance.

BACKGROUND: The Ponseti method for treating congenital talipes equinovarus requires an orthosis to maintain correction after manipulation and casting, typically the 'boots and bar'. Non-compliance with the orthosis increases the risk of recurrent deformity. This study investigates a new orthosis, the abduction dorsiflexion mechanism. OBJECTIVES: The aim of this study is to assess compliance of the abduction dorsiflexion mechanism when used at night and maintenance of foot morphology. STUDY DESIGN: This study is a cohort study. METHODS: A total of 10 children with unilateral congenital talipes equinovarus previously treated with Ponseti casting were recruited to trial the abduction dorsiflexion mechanism at night for 12 weeks. Foot morphology and compliance were assessed every 4 weeks. RESULTS: Participant families were pleased with the orthosis, opting to continue to use the device following conclusion of the trial. Compliance was good and no deterioration in Pirani score or dorsiflexion seen. Abduction improved during the trial. The incidence of skin problems was equivalent to that experienced with the traditional boots and bar that the children had been wearing. CONCLUSION: Good compliance and parental satisfaction, coupled with no deterioration in foot morphology, abduction or dorsiflexion present the abduction dorsiflexion mechanism boot as a feasible alternative to the traditional boots and bar, particularly in children with unilateral congenital talipes equinovarus. CLINICAL RELEVANCE: The abduction dorsiflexion mechanism is currently the subject of considerable interest as clinicians look to increase compliance and reduce the recurrence rate in Ponseti-treated congenital talipes equinovarus. The abduction dorsiflexion mechanism boot is a feasible alternative to the traditional boots and bar, particularly in children with unilateral congenital talipes equinovarus.

Spinal cord infarction in a sick neonate from predominant haemorrhagic aetiology: a case report.

INTRODUCTION: Spinal cord injuries in new born infants following a traumatic delivery or umbilical cord catheterisation due to thromboembolism are well known. Cases with atraumatic acute onset of neonatal paraplegia have also been described in preterm babies or babies born small for gestational age with a stormy postnatal course related to ischaemic aetiology. We describe a rare case of infarction of the spinal cord from a predominant haemorrhagic aetiology. CASE PRESENTATION: A term female baby, first child of unrelated parents, was born by normal vaginal delivery. She had meconium aspiration at birth, leading to severe respiratory distress, requiring neonatal intensive care admission. At 2 weeks, she developed new flaccid paraplegia. MRI scan of the spine showed haemorrhagic infarction of the spinal cord from the level of thoracic inlet, vertebral level C7-T1. A follow-up MRI scan at 11 months revealed severe atrophy of the cord distal to C6. At 3 years of age, she had good upper-limb function, diaphragmatic breathing and flaccid paralysis of lower limbs. DISCUSSION: In an acutely unwell term infant with symptoms of paralysis or spinal cord damage, haemorrhagic infarction needs to be considered in the differential diagnosis. To our knowledge, this is the first reported case of spinal cord injury in a term infant with a haemorrhagic lesion, and it helps to understand the pathogenesis of nontraumatic insult.

Outcome of the unaffected contralateral hip in unilateral slipped capital femoral epiphysis: a report comparing prophylactic fixation with observation.

This was a retrospective review of 80 patients with unilateral slipped capital femoral epiphysis treated with either prophylactic fixation or observation. The unaffected hip of 44 patients (mean age 12.6 years) were subjected to simultaneous prophylactic fixation and 36 patients (mean age 13.4 years) were managed by observation. Fisher's exact test showed a significantly high incidence of sequential slip of unaffected hips in the observation group in comparison with prophylactic fixation (P=0.002). No cases had avascular necrosis or chondrolysis. Prophylactic fixation significantly reduces the incidence of sequential slip. It outweighs the minimal surgical risks compared with its benefit in the prevention of further sequential slip.

Ossification of the femoral head at closed reduction for developmental dysplasia of the hip and its influence on the long-term outcome.

The management of developmental dysplasia of the hip, particularly the timing of closed reduction with regard to the presence of the femoral head ossific nucleus, is controversial. The clinical and radiological outcome was assessed in a strictly defined group of 48 hips in 42 patients with an average of 11.1 years follow-up. One hundred percent of patients had an excellent or good result at final follow-up; 8.3% showed evidence of avascular necrosis and six hips underwent further surgery after closed reduction. There was no relationship between the presence or absence of an ossific nucleus at the time of closed reduction and the final outcome. In this well-defined group, closed reduction is safe and provides excellent results in the long term.

Ponseti treatment in the management of clubfoot deformity - a continuing role for paediatric orthopaedic services in secondary care centres.

INTRODUCTION: The Ponseti technique is a well-proven way of managing paediatric clubfoot deformity. We describe a management set-up which spreads the care between secondary and tertiary care with no loss of quality. PATIENTS AND METHODS: In our audit of the first 2 years of Ponseti casting in the treatment of idiopathic congenital talipes equinovarus (CTEV, clubfoot) deformity, we identified 77 feet having been treated in 50 patients. Forty-nine feet were treated primarily in Oswestry, a tertiary referral centre for paediatric orthopaedic conditions, and 13 feet were treated in conjunction with the physiotherapy department at one of the region's district general hospitals (Leighton Hospital, Crewe, Cheshire). RESULTS: Similar good results and low requirement for surgical interventions other than Achilles tenotomy, which forms part of the Ponseti regimen, were found in both cohorts. CONCLUSIONS: This 'hub-and-spoke' approach would appear to be efficient in terms of resource utilisation. Additional benefits for patients and their carers include ease of access to services and reduced financial and transport burdens.

Mechanical properties of different combinations of flexible nails in a model of a pediatric femoral fracture.

Flexible nailing of pediatric femoral shaft fractures is based on the principle of using two C-shaped nails to create six points of fixation. However, clinical studies using various nail combinations demonstrate similar outcomes. This study aimed to compare the mechanical properties of different nail combinations by testing them in a model of a child's midshaft femoral fracture. The two C-shaped nails were compared with two straight nails and with paired S- and C-shaped nails. The constructs were tested in four-point bending and torsion. Graphs of the data were produced, from which the bending and torsional stiffness of the constructs was calculated. The results showed that there was no significant difference between the mechanical properties of the three different constructs. The conclusion is that any of the tested nail combinations can be used to treat a midshaft fracture of the femur in a child.